Document Detail

Hepatic peptidyl prolyl hydroxylase activity and liver fibrosis--a prospective study of 94 infants and children with hepatobiliary disorders.
MedLine Citation:
PMID:  6327486     Owner:  NLM     Status:  MEDLINE    
To assess whether hepatic peptidyl prolyl hydroxylase (PPH) activity could serve as a practical quantitative indicator of hepatic fibrosis or aid in the categorization, diagnosis or prognosis of hepatobiliary disorders in infancy and childhood, the activity of this enzyme has been determined prospectively by a tritium release method in 97 biopsies from 94 infants and children with the following conditions: acute hepatitis of infancy, 10 patients; extrahepatic biliary atresia, 13; previous hepatitis of infancy, 8; alpha-1-antitrypsin deficiency, 6; chronic active hepatitis, 17; chronic persistent hepatitis, 5; glycogen storage disease, 5; and 25 patients with a miscellanea of other liver disorders. PPH activity was considered in relation to diagnosis, biochemical and histological abnormality and subsequent prognosis over a 4-year period. Five liver biopsies which showed no histological abnormality were considered as "controls" having PPH values of 0.72 +/- 0.47 (mean +/- S.D.). PPH activity was significantly elevated in acute hepatitis of infancy, 9 of the 10 infants having PPH greater than 1.66 units (i.e., mean +/- 2 S.D. of the "control" value). Nine infants (70%) with extrahepatic biliary atresia also had PPH activity above this value, as did two with alpha-1-antitrypsin deficiency and 12 patients all in different diagnostic categories. PPH activity did not correlate with hepatic fibrosis as indicated by hepatic hydroxyproline concentration or by histological assessment, or with biochemical tests of liver function within any diagnostic group or in the series as a whole. PPH activity was similar in biopsies with and without histological features of cirrhosis.(ABSTRACT TRUNCATED AT 250 WORDS)
P Trivedi; M S Tanner; B Portmann; J McClement; A P Mowat
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Hepatology (Baltimore, Md.)     Volume:  4     ISSN:  0270-9139     ISO Abbreviation:  Hepatology     Publication Date:    1984 May-Jun
Date Detail:
Created Date:  1984-07-13     Completed Date:  1984-07-13     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  8302946     Medline TA:  Hepatology     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  436-41     Citation Subset:  IM    
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MeSH Terms
Bile Ducts / abnormalities*
Child, Preschool
Cholestasis, Intrahepatic / enzymology
Collagen / analysis
Hepatitis / enzymology
Liver / enzymology*
Liver Cirrhosis / enzymology
Liver Diseases / enzymology*
Liver Neoplasms / enzymology
Procollagen-Proline Dioxygenase / analysis*
Prospective Studies
alpha 1-Antitrypsin Deficiency
Reg. No./Substance:
9007-34-5/Collagen; EC Dioxygenase

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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